title: Apoptosis status and proliferative activity in mucopolysaccharidosis type I mice tongue mucosa cells.
abstract: BACKGROUND
  Mucopolysaccharidosis type I (MPS I) is caused by a deficiency of alfa-iduronidase (IDUA), which leads to intralysosomal accumulation of glysosaminoglycans. Evidences point secondary events like oxidative stress on lysosomal storage diseases including MPS I. Patients with MPS I present a wide range of oral clinical manifestations, including tongue hypertrophy, hypertrophyc alveolar process, and carious teeth. However, the mechanisms by which these alterations occur are still not fully understood. The aim of this study was to analyze the proliferative activity as well as apoptosis in tongue mucosa cells from murine model of MPS I.
  
  
  METHODS
  Protein expression of apoptotic markers such as p53, bcl-2 and bax were evaluated in this setting. Ki-67 was used as a proliferative marker. All analyses were made by immunohistochemistry in tongue cells. Statistical analysis was perfomed by Kruskal-Wallis non-parametric test followed by the Dunn's test. P < 0.05 was considered for statistic significance.
  
  
  RESULTS
  Histopathological analysis revealed no remarkable differences in tongue mucosa on MPS I mice when compared to control. By contrast, our results demonstrated that bcl-2 immunoexpression was decreased in mice tongue mucosa cells of MPS I mice. p53, bax and ki-67 immunoexpresssion did not show significant differences among controls and MPS I mice.
  
  
  CONCLUSIONS
  Taken together, our results suggest that IDUA deficiency, which characterizes MPS I, may induce apoptosis in mice tongue cells as a result of bcl-2 down regulation.
authors.affiliation: Department of Pathology, Federal University of São Paulo, UNIFESP, SP, Brazil.
authors.forename: Juliana
authors.initials: J
authors.lastname: Noguti
authors.forename: Vanessa Gonçalves
authors.initials: VG
authors.lastname: Pereira
authors.forename: Joice Marques
authors.initials: JM
authors.lastname: Guilheiro
authors.forename: Ana Maria
authors.initials: AM
authors.lastname: Martins
authors.forename: Vânia
authors.initials: V
authors.lastname: D'Almeida
authors.forename: Daniel Araki
authors.initials: DA
authors.lastname: Ribeiro
keywords: Apoptosis; bax; bcl-2; ki-67 mucopolysaccharidosis; p53; tongue mucosa cells